Recurrent cerebral arteriovenous malformations after negative postoperative angiograms

Abstract

Angiography has been considered to be the gold standard to judge the success of treatment for cerebral arteriovenous malformations (AVMs). Patients without residual nidus or early draining veins on postoperative angiograms are considered cured, with the risk of hemorrhage eliminated. A series of five patients with recurrent AVMs after negative postoperative angiography is described. All patients had hemispheric AVMs, presented initially with hemorrhage, and were between 5 and 13 years of age. Recurrence was noted 1 to 9 years later (at 12-16 years of age); after a hemorrhage in three patients, seizures in one, and on follow-up magnetic resonance imaging in one. Four patients underwent angiography that showed recurrence of the AVM at or adjacent to the original site. Three years postsurgery, the fifth patient died from a large intracerebral and intraventricular hemorrhage originating in the previous location of the AVM; however, the patient did not undergo angiography at the time of recurrence. The initial negative angiograms obtained postoperatively in these patients may be explained by postoperative spasm or thrombosis of a small residual malformation. However, in the authors' cumulative experience with 808 patients who have undergone complete surgical removal of AVMs (of whom 667 were older than 18 years of age), no case of recurrent AVM has been observed in an adult. Therefore, actual regrowth of an AVM may occur in children and could be a consequence of their relatively immature cerebral vasculature and may involve active angiogenesis mediated by humoral factors. The present findings argue against the assumption that AVMs are strictly congenital lesions resulting from failure of capillary formation during early embryogenesis. It is concluded that delayed imaging studies should be considered in children at least 1 year after their initial negative postoperative arteriogram to exclude a recurrent AVM.

Nestin Expression in Vascular Malformations: A Novel Marker for Proliferative Endothelium

“…17) However, clinical studies have detected growth and de novo appearance of vascular malformations. 3,9,15,18,20) The underlying developmental and pathogenetic mechanisms of these malformations are not completely understood, but may involve the proliferative and angiogenic capacities of the endothelium. 7,13,21,23) Nestin is one of the intermediate filaments abundantly produced in the developing central nervous system and somites in the embryonic stage of rodents, and is used as a marker of neuroepithelial stem cells.…”

Increased expression of ephrin A1 in brain arteriovenous malformation: DNA microarray analysis

“…The increasing size of AVMs over time has been documented in the literature [26]. Recurrent AVMs after negative angiograms have also been reported [13]. It is well known that new shunts between arteries and veins grow after embolization of feeding arteries.…”

“…They are generally believed to arise when capillaries fail to develop in an area of the brain during early embryogenesis, resulting in abnormal communications between arteries and veins. The growth of blood vessels during embryogenesis and in adult organisms is tightly controlled, and this growth is thought to be mediated by soluble factors [13,19,27]. A number of previous studies revealed the abnormal expression of various angiogenesis-related genes or products in brain AVM [7-11, 14, 16, 27].…”

Surgical Treatment of Non-ruptured Giant Occipital Arteriovenous Malformations With Frequent Migraine-Like Headache-Two Case Reports-

“…19) Previous cases of AVM recurrence after surgical excision and postoperative angiographic disappearance suggest that regrowth of the remnant arteriovenous shunt will result in development of de novo AVM nidus. 2,7) We speculate that development of the dural feeders might further enhance gradual growth of the nidus, resulting in homonymous hemianopia. Giant occipital AVMs might enlarge beyond the extent of the occipital lobe, becoming inoperable and incurable, and as a result, the severe migraine-like headaches and AVM will persist.…”